Positive-Pressure Ventilation in Amyotrophic Lateral Sclerosis

  1. Marcia Bio, MD; and
  2. John R. Bach, MD
  1. University of Medicine and Dentistry of New Jersey Medical School; Newark, NJ 07103

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    TO THE EDITOR:

    Although Aboussouan and colleagues [1] reaffirm that assisting the ventilation of patients with amyotrophic lateral sclerosis can prolong survival, even their patients who “tolerated” nocturnal nasal positive-pressure ventilation survived only 15 months. Most untreated patients with this disease die of CO2 narcosis or inability to clear airway secretions [2]. However, patients for whom assisted peak cough flows greater than 160 L/min can be generated (that is, patients with less bulbar involvement) and who are equipped and trained in using assisted coughing, mechanical insufflation-exsufflation, and up to 24-hour non-invasive positive-pressure ventilation as needed can have prolonged survival without tracheostomy [3]. In contrast, those with assisted peak cough flows less than 160 L/min will require tracheostomy [3].

    Aboussouan and colleagues prevented neither daytime hypoventilation with a mouth piece or nasal positive-pressure ventilation nor respiratory failure from bronchial mucus plugging. They noted that mechanical insufflation-exsufflation was used but did not describe how or when this procedure was done. This procedure is generally not effective when severe bulbar dysfunction results in assisted cough flows less than 160 L/min [4]. Furthermore, the tidal volumes used by the authors were insufficient to compensate for air leakage in this open system, and bulbar function assessment was based on an arbitrary scale rather than on peak cough flow [3, 4]. Thus, the authors underestimated the potential of noninvasive aids to prolong survival. Because we maintain alveolar ventilation around the clock and create effective cough flows, some of our patients with amyotrophic lateral sclerosis have required 24-hour ventilatory support for many years but have never undergone intubation, tracheostomy, or even hospitalization [3]. We recommend that all who are interested in this strategy read more on the subject [5].

    Marcia Bio, MD

    John R. Bach, MD

    University of Medicine and Dentistry of New Jersey Medical School; Newark, NJ 07103

    The Editors welcome submissions for possible publication in the Letters section. Authors of letters should:

    •Include no more than 300 words of text, three authors, and five references

    •Type with double-spacing

    •Send three copies of the letter, an authors' form signed by all authors, and a cover letter describing any conflicts of interest related to the contents of the letter.

    Letters commenting on an Annals article will be considered if they are received within 6 weeks of the time the article was published. Only some of the letters received can be published. Published letters are edited and may be shortened; tables and figures are included only selectively. Authors will be notified that the letter has been received. If the letter is selected for publication, the author will be notified about 3 weeks before the publication date. Unpublished letters cannot be returned.

    Annals welcomes electronically submitted letters.

    References

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